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Article

FUS unveiled in mitochondrial DNA repair and targeted ligase-1 expression rescues repair-defects in FUS-linked motor neuron disease.

Nature communications - 9 Mar 2024

Kodavati Manohar, Wang Haibo, Guo Wenting, Mitra Joy, Hegde Pavana M, Provasek Vincent, Rao Vikas H Maloji, Vedula Indira, Zhang Aijun, Mitra Sankar, Tomkinson Alan E, Hamilton Dale J, Van Den Bosch Ludo, Hegde Muralidhar L

Abstract excerpt

This study establishes the physiological role of Fused in Sarcoma (FUS) in mitochondrial DNA (mtDNA) repair and highlights its implications to the pathogenesis of FUS-associated neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS). Endogenous FUS interacts with and recruits mtDNA Ligase IIIα (mtLig3) to DNA damage sites within mitochondria, a relationship essential for maintaining mtDNA repair...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 38461154
DOI: 10.1038/s41467-024-45978-6
PMCID: PMC10925063

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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